EVALUATION OF THE EFFECTIVENESS OF DENTAL REHABILITATION OF CHILDREN WITH ORPHAN DISEASES AND DISORDERS OF PHOSPHORUS-CALCIUM METABOLISM BASED ON QUALITY OF LIFE CRITERIA

In recent decades, quality of life assessment has become an integral part of a comprehensive approach to managing patients with rare diseases. An integrated assessment of the impact of dental health on key areas of life is becoming a component of comprehensive rehabilitation for children with rare diseases, aimed at restoring quality of life. Objective: To study the impact of dental rehabilitation on the dynamics of quality of life criteria in children with rare phosphorus-calcium metabolism disorders using the OHRQoL questionnaire. Materials and Methods A clinical and sociological study was conducted in groups of children (6–17 years) with the genetic diseases X-linked hypophosphatemic rickets (X-HPR), hypophosphatasia (HPP), and osteogenesis imperfecta (OI). The effects of dental rehabilitation on the condition of dental tissues in children were studied according to clinical indices (OHI-S, CAST) and the dynamics of quality of life criteria using the Oral Health-Related Quality of Life (OHRQoL) parental questionnaire. The questionnaire (OHRQoL) is a tool for assessing the impact of dental problems on various areas of daily life, reflecting physical discomfort in the oral cavity; the child's communication difficulties; support and resolution of dental problems by legal representatives. Results. Dental rehabilitation in groups of children with X-linked hypophosphatemic rickets (X-HPR), hypophosphatasia (HPP), and osteogenesis imperfecta (OI) demonstrated significant clinical efficacy and a reduction in physical discomfort in the oral cavity, as evidenced by a reduction in the functional domain scores of the OHRQoL questionnaire. Furthermore, it was found that parents of children with orphan diseases showed increased adherence to dental treatment and preventive measures.